new jersey critical congenital heart defects screening program
TRANSCRIPT
Microsoft PowerPoint - Rob Koppel - Pulse Ox presentation
[Read-Only]New Jersey Critical Congenital Heart Defects Screening
Program
Keeping the Beat: Pediatric Cardiac Screening and Management of Congenital Heart Disease
Prenatal Diagnosis of Critical Congenital Heart Disease
Robert Koppel, MD Neonatal/Perinatal Medicine, Pediatrics
North Shore-LIJ Medical Group Associate Professor
Hofstra North Shore-LIJ School of Medicine
March 24, 2015
Objectives
Recognize the importance of screening for Critical Congenital Heart Defects (CCHD) using pulse oximetry
Apply the New Jersey recommended CCHD screening protocol
Identify the role of the primary care physician in the detection of and referral of CCHD
New Jersey Critical Congenital Heart Defects Screening Program
Disclosure
Dr. Koppel has no conflict of interest to disclose.
The New Jersey Department of Health does not endorse or promote a specific brand or vendor for pulse oximetry supplies and equipment. Equipment and/or supplies presented in the education are for informational purposes only.
New Jersey Critical Congenital Heart Defects Screening Program
The Law
“The Commissioner of Health and Senior Services shall require each birthing facility licensed by the Department of Health and Senior Services to perform a pulse oximetry screening, a minimum of 24 hours after birth, on every newborn in its care.”
NJ first state to implement a mandate for pulse oximetry screening
Legislation signed into law June 2, 2011
Implementation date August 31, 2011
P.L. 2011, Chapter 74
Congenital Heart Defects
Minimum of 32,000 40,000 infants affected each year in US
Approximately 25% of these are critical congenital heart defects (CCHD) or about 2 in 1,000 live births
New Jersey Critical Congenital Heart Defects Screening Program
Dylan was transferred to Columbia University Medical Center, and several days later had the lifesaving surgery correcting the abnormality discovered from the newly mandated newborn testing.
Dylan’s Story
On September 1, a day after the law mandating inclusion of pulse oximetry testing on newborns became effective, a hospital pediatrician informed Lisa and Bill Gordon of Newton that the test performed on their baby was abnormal and he had a murmur.
Dylan was rushed to Morristown Medical Center, where it was determined he needed specialized pediatric cardiac heart surgery.
http://www.state.nj.us/governor/news/news/552011/approved/20111109a.html
2
Rationale for Pulse Ox Screening
An estimated 2530% of newborns with CCHD could be missed at the time of hospital discharge (Mahle et. al., 2009)
About 1,200 more newborns with CCHD could be identified at birth hospitals using pulse oximetry (Peterson et al., 2013.)
Approximately 200 newborns have died each year from missed CCHD and numerous others have significant morbidity from delayed diagnoses (Hokanson, 2010.)
Compare to an average 66 young athletes each year who die suddenly of undiagnosed cardiac defects (Maron et al. 2009)
New Jersey Critical Congenital Heart Defects Screening Program
Shock global hypoxemic injury with multi organ dysfunction
• Hypotension • Poor ventricular function • Myocardial ischemia • Pulmonary hypertension • Renal dysfunction • Hepatic dysfunction • Decreased intestinal blood flow NEC • DIC • Metabolic: hypoglycemia, hypocalcemia, myoglobinuria, hypoxicischemic encephalopathy
Mahle et al., 2009.
New Jersey Critical Congenital Heart Defects Screening Program
Detection of CCHD Continuum
Detection of CCHD
Not As Pink As You Think…
Some babies can initially appear healthy Some babies do not have murmurs or cyanosis
Physical exam alone failed to identify half of CHDs that were not detected by a prenatal ultrasound
It’s estimated that 30% of infant deaths from CCHD occur prior to diagnosis
New Jersey Critical Congenital Heart Defects Screening Program
The Cyanotic “Blind Spot”
CCHD Screening with Pulse Oximetry
Indirectly monitors the oxygen saturation of a patient's blood and variations in blood flow in the skin
Can detect mild hypoxemia without apparent cyanosis
Can provide continuous and direct values Noninvasive Easy to use and widely available Costeffective and extensively used
The Texas Pulse Oximetry Project, 2013.
New Jersey Critical Congenital Heart Defects Screening Program
Newborn Screening New York – 1960’s
Robert Guthrie, MD Virginia Apgar, MD
Source: WikipediaSource: Museum of Disability History
New Jersey Critical Congenital Heart Defects Screening Program
Screening Cost
CDC Study in 7 NJ Birthing facilities Mean screening time per newborn was 9.1 (standard deviation 3.4 minutes)
Hospitals’ total mean estimated cost per newborn screened was $14.19 (in 2011 U.S. dollars), consisting of $7.36 in labor costs and $6.83 in equipment and supply costs
Peterson et al., 2014.
Screening Cost per Infant
Laboratory Metabolic Screening $20.00
Pulse Ox Screening $14.19
These cost estimates exclude follow-up costs, such as further diagnostic testing, as well as administrative overhead costs.
New Jersey Critical Congenital Heart Defects Screening Program
Screening Case Discussion
HLHS not screened; late detection
Coarctation of aorta passed screen (false negative); late detection
New Jersey Critical Congenital Heart Defects Screening Program
Case Presentation
Discharged home on day 3
Day 5: returned to ED for poor feeding and decreased activity
SpO2: 80%
Norwood stage I
4
Case Presentation
39 weeks, NSVD, Apgar 9/9
Discharged home on Day 2 Oximetry screening postductal SpO2 100%
Day 3 Lethargy Decreased PO intake Dry diapers Tachypnea Evaluated by pediatrician
New Jersey Critical Congenital Heart Defects Screening Program
Case Presentation
Referral to ED for respiratory distress grunting retracting unable to measure SpO2
Intubated
ABG: 7.09/17/199/8/23.3
Echo: coarctation, ductus arteriosus closed (history of normal fetal echo)
Prostaglandin infusion
New Jersey Critical Congenital Heart Defects Screening Program
CCHD Screening
Seven Primary Targets (1731% of all CHDs): Hypoplastic left heart syndrome
Pulmonary atresia (with intact ventricular septum)
Tetralogy of Fallot
Tricuspid atresia
Truncus arteriosus
CCHD Screening
Double outlet right ventricle
Effect of CCHD on O2 Saturations
Modified diagrams courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.
www.cdc.gov/ncbddd/heartdefects
Effect of CCHD on O2 Saturations
Modified diagrams courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.
www.cdc.gov/ncbddd/heartdefects
5
Thangaratinam et al., 2012.
Reliability of Pulse Ox Screening for CCHD
Metaanalysis of 13 eligible studies with data for 229,421 newborn babies Overall sensitivity of pulse oximetry for detection of critical congenital heart defects was 76.5% (95% CI 67.7 – 83.5)
Specificity was 99.9% (95% CI 99.7 – 99.9) Falsepositive rate of 0.14% (95% CI 0.06 – 0.33) Low false positive rate when pulse ox done after 24 hours from birth compared to before 24 hours
New Jersey Critical Congenital Heart Defects Screening Program
CCHD Screening 2011
Indiana Law Enacted
1 st Newborn
Kemper Pediatrics Paper
New Jersey Law Enacted
AAP CCHD Screening Map: 2011 State Actions on CCHD
3 states enacted legislation
New Jersey Critical Congenital Heart Defects Screening Program
AAP CCHD Screening Map: States’ Actions During 2014 (As of January 29, 2015)
New Jersey Critical Congenital Heart Defects Screening Program
Performing the Screen
Best results when infant is at least 24 hours of age
Use proper sensor for the device
Conduct screening in quiet area and, if possible, with parent present to soothe and comfort the infant
Conduct screening while infant is awake and quiet
Avoid screening when infant is crying, cold or in a deep sleep
New Jersey Critical Congenital Heart Defects Screening Program
Reich et al., 2008.
Motion artifact
Ambient light
NJ Recommended Algorithm: Abridged Version
95%100% in both extremities AND a difference of 3% or less between the readings. 95%100% in both extremities AND a difference of 3% or less between the readings.PASS
90%94% in either extremity OR a difference of 4% or more between the readings. Rescreen the infant up to 2 times, for a total of 3 screens.
90%94% in either extremity OR a difference of 4% or more between the readings. Rescreen the infant up to 2 times, for a total of 3 screens.
RESCREEN IN 1 HOUR
90%94% in either extremity OR a difference of 4% or more between the readings after three screens.
90%94% in either extremity OR a difference of 4% or more between the readings after three screens.
FAIL
A reading of 89% or less in either extremity.A reading of 89% or less in either extremity. IMMEDIATE
FAIL
Evaluation for Failed Screen
Complete echocardiogram
New Jersey Critical Congenital Heart Defects Screening Program
Managing the Failed Screen
Unless a noncardiac cause can be identified for a failed screen, an infant who fails the screen should have a diagnostic echocardiogram done before being discharged
This could involve an echocardiogram within the hospital or birthing center, transport to another institution for the procedure, or the use of telemedicine for remote evaluation
New Jersey Critical Congenital Heart Defects Screening Program
Parent Education
Communication of Screening Results
Include screening results in discharge summary
Include in the handoff report to the receiving hospital if infant was transferred
Pediatrician
Early Intervention
Early Care and Education
First Office Visit
Pediatrician should have access to all screening results from hospital (Metabolic, CCHD, Hearing)
This should be a “clean handoff” between the hospital and pediatrician
If patient not appropriately screened at birth facility, develop strategies for screening and evaluation
New Jersey Critical Congenital Heart Defects Screening Program
Oster, 2013.
If Your Patient Failed CCHD Screening
Confirm that the infant had a diagnostic echocardiogram Make sure that the patient receives appropriate
followup, such as being seen by a cardiologist
Facilitate longterm followup for patients diagnosed with CCHDs
New Jersey Critical Congenital Heart Defects Screening Program
Signs & Symptoms of CHD
Lethargy
New Jersey Pulse Ox Screening Experience
8
New Jersey POxS Experience
Data Reporting Birthing facilities are required to report all failed POxS to the NJ Birth Defects Registry (BDR)
Health care professionals are required to report infants with CCHD (and other congenital defects) who are New Jersey residents to the BDR
Pulse ox core team and BDR staff investigate all POxS fails and registered cases of CCHD
New electronic birth record (Vital Information Platform) includes prenatal CCHD detection and POxS data
New Jersey Critical Congenital Heart Defects Screening Program
Results of NJ Screening
Number of livebirths: 313,005
Number of livebirths eligible to be screened: 304,211
Number of livebirths screened: 303,090
Proportion of eligible livebirths screened: 99.6 %
*Excludes expirations, <24 hours or not medically appropriate at end of period*Excludes expirations, <24 hours or not medically appropriate at end of period
New Jersey Critical Congenital Heart Defects Screening Program
Failed Screens Registered to NJBDR: August 31, 2011 September 30, 2014
One of 3 Criteria Prenatal diagnosis of
CCHD
Cardiac consult or echocardiogram planned prior to the screen
Total fails N= 208
N= 97
N= 111
New Jersey Critical Congenital Heart Defects Screening Program
Failed Screens Registered to NJBDR
Diagnostic evaluation attributable to POxS (n=97) 14 CCHD
12 CHD
27 PDA or PFO as only finding
35 – No documented reason for failed POxS 23 (66%) did not follow protocol
New Jersey Critical Congenital Heart Defects Screening Program
14 Infants with CCHD Were Detected
Coarctation of the aorta (5)
Ebstein anomaly (1)
Tricuspid atresia (1)
New Jersey Critical Congenital Heart Defects Screening Program
Characteristics of Failed Screens: CCHD CASE AGE AT
SCREEN PRE DUCTAL
FINAL DIAGNOSIS
1 2 DAYS 97 84 1 Y Y COARCTATION OF AORTA
2 3 DAYS 94 86 1 Y Y COARCTATION OF AORTA
3 2 DAYS 99 88 1 Y Y COARCTATION OF AORTA Out of state resident
4 2 DAYS 99 90 1 N Y COARCTATION OF AORTA
5 3 DAYS 92 97 3 Y Y COARCTATION OF AORTA* NICU
6 2 DAYS 43 39 1 Y Y DTGA
7 2 DAYS 85 85 1 Y Y DTGA
8 2 DAYS 87 84 1 Y Y TAPVR
9 2 DAYS 92 93 2 N Y TAPVR
10 2 DAYS 77 72 3 N Y TAPVR
11 3 DAYS 89 93 1 Y Y TAPVR
12 2 DAYS 85 88 1 Y Y TAPVR
13 2 DAYS 91 92 1 N Y TRICUSPID ATRESIA
14 3 DAYS 95 92 3 Y N EBSTEIN ANOMALY Passed national protocol
9
Other Conditions Detected
12 CHDs: Atrial septal aneurysm ASD VSD Pulmonary artery/pulmonary artery branch stenosis
9 Other significant nonCHD medical conditions: Sepsis Pneumonia Persistent pulmonary hypertension Pulmonary bulla
New Jersey Critical Congenital Heart Defects Screening Program
Screening Success
Since implementation, 99.6% of eligible infants were screened in New Jersey
POxS in NJ led to the detection of: 14 infants with critical congenital heart defects (CCHD)
12 infants with other congenital heart disease (CHD)
9 infants with serious noncardiac conditions
New Jersey Critical Congenital Heart Defects Screening Program
Acknowledgements
Lori Garg, MD, MPH
Mary Knapp, MSN, RN
Kim Van Naarden Braun, PhD
New Jersey Chapter, American Academy of Pediatrics
Regina Grazel, MSN, RN, BC, APNC
Fran Gallagher, MEd
Harriet Lazarus, MBA
Lindsay Caporrino, BS
Contact Information Regina Grazel, MSN, RN, BC, APNC
Project Coordinator, NJ DOH CCHD Screening Program [email protected]
[email protected]
American Academy of Pediatrics http://www.aap.org/enus/advocacyandpolicy/aaphealth initiatives/PEHDIC/Pages/NewbornScreeningforCCHD.aspx
New Jersey Chapter, American Academy of Pediatrics http://www.aapnj.org/showcontent.aspx?MenuID=1627
Keeping the Beat: Pediatric Cardiac Screening and Management of Congenital Heart Disease
Prenatal Diagnosis of Critical Congenital Heart Disease
Robert Koppel, MD Neonatal/Perinatal Medicine, Pediatrics
North Shore-LIJ Medical Group Associate Professor
Hofstra North Shore-LIJ School of Medicine
March 24, 2015
Objectives
Recognize the importance of screening for Critical Congenital Heart Defects (CCHD) using pulse oximetry
Apply the New Jersey recommended CCHD screening protocol
Identify the role of the primary care physician in the detection of and referral of CCHD
New Jersey Critical Congenital Heart Defects Screening Program
Disclosure
Dr. Koppel has no conflict of interest to disclose.
The New Jersey Department of Health does not endorse or promote a specific brand or vendor for pulse oximetry supplies and equipment. Equipment and/or supplies presented in the education are for informational purposes only.
New Jersey Critical Congenital Heart Defects Screening Program
The Law
“The Commissioner of Health and Senior Services shall require each birthing facility licensed by the Department of Health and Senior Services to perform a pulse oximetry screening, a minimum of 24 hours after birth, on every newborn in its care.”
NJ first state to implement a mandate for pulse oximetry screening
Legislation signed into law June 2, 2011
Implementation date August 31, 2011
P.L. 2011, Chapter 74
Congenital Heart Defects
Minimum of 32,000 40,000 infants affected each year in US
Approximately 25% of these are critical congenital heart defects (CCHD) or about 2 in 1,000 live births
New Jersey Critical Congenital Heart Defects Screening Program
Dylan was transferred to Columbia University Medical Center, and several days later had the lifesaving surgery correcting the abnormality discovered from the newly mandated newborn testing.
Dylan’s Story
On September 1, a day after the law mandating inclusion of pulse oximetry testing on newborns became effective, a hospital pediatrician informed Lisa and Bill Gordon of Newton that the test performed on their baby was abnormal and he had a murmur.
Dylan was rushed to Morristown Medical Center, where it was determined he needed specialized pediatric cardiac heart surgery.
http://www.state.nj.us/governor/news/news/552011/approved/20111109a.html
2
Rationale for Pulse Ox Screening
An estimated 2530% of newborns with CCHD could be missed at the time of hospital discharge (Mahle et. al., 2009)
About 1,200 more newborns with CCHD could be identified at birth hospitals using pulse oximetry (Peterson et al., 2013.)
Approximately 200 newborns have died each year from missed CCHD and numerous others have significant morbidity from delayed diagnoses (Hokanson, 2010.)
Compare to an average 66 young athletes each year who die suddenly of undiagnosed cardiac defects (Maron et al. 2009)
New Jersey Critical Congenital Heart Defects Screening Program
Shock global hypoxemic injury with multi organ dysfunction
• Hypotension • Poor ventricular function • Myocardial ischemia • Pulmonary hypertension • Renal dysfunction • Hepatic dysfunction • Decreased intestinal blood flow NEC • DIC • Metabolic: hypoglycemia, hypocalcemia, myoglobinuria, hypoxicischemic encephalopathy
Mahle et al., 2009.
New Jersey Critical Congenital Heart Defects Screening Program
Detection of CCHD Continuum
Detection of CCHD
Not As Pink As You Think…
Some babies can initially appear healthy Some babies do not have murmurs or cyanosis
Physical exam alone failed to identify half of CHDs that were not detected by a prenatal ultrasound
It’s estimated that 30% of infant deaths from CCHD occur prior to diagnosis
New Jersey Critical Congenital Heart Defects Screening Program
The Cyanotic “Blind Spot”
CCHD Screening with Pulse Oximetry
Indirectly monitors the oxygen saturation of a patient's blood and variations in blood flow in the skin
Can detect mild hypoxemia without apparent cyanosis
Can provide continuous and direct values Noninvasive Easy to use and widely available Costeffective and extensively used
The Texas Pulse Oximetry Project, 2013.
New Jersey Critical Congenital Heart Defects Screening Program
Newborn Screening New York – 1960’s
Robert Guthrie, MD Virginia Apgar, MD
Source: WikipediaSource: Museum of Disability History
New Jersey Critical Congenital Heart Defects Screening Program
Screening Cost
CDC Study in 7 NJ Birthing facilities Mean screening time per newborn was 9.1 (standard deviation 3.4 minutes)
Hospitals’ total mean estimated cost per newborn screened was $14.19 (in 2011 U.S. dollars), consisting of $7.36 in labor costs and $6.83 in equipment and supply costs
Peterson et al., 2014.
Screening Cost per Infant
Laboratory Metabolic Screening $20.00
Pulse Ox Screening $14.19
These cost estimates exclude follow-up costs, such as further diagnostic testing, as well as administrative overhead costs.
New Jersey Critical Congenital Heart Defects Screening Program
Screening Case Discussion
HLHS not screened; late detection
Coarctation of aorta passed screen (false negative); late detection
New Jersey Critical Congenital Heart Defects Screening Program
Case Presentation
Discharged home on day 3
Day 5: returned to ED for poor feeding and decreased activity
SpO2: 80%
Norwood stage I
4
Case Presentation
39 weeks, NSVD, Apgar 9/9
Discharged home on Day 2 Oximetry screening postductal SpO2 100%
Day 3 Lethargy Decreased PO intake Dry diapers Tachypnea Evaluated by pediatrician
New Jersey Critical Congenital Heart Defects Screening Program
Case Presentation
Referral to ED for respiratory distress grunting retracting unable to measure SpO2
Intubated
ABG: 7.09/17/199/8/23.3
Echo: coarctation, ductus arteriosus closed (history of normal fetal echo)
Prostaglandin infusion
New Jersey Critical Congenital Heart Defects Screening Program
CCHD Screening
Seven Primary Targets (1731% of all CHDs): Hypoplastic left heart syndrome
Pulmonary atresia (with intact ventricular septum)
Tetralogy of Fallot
Tricuspid atresia
Truncus arteriosus
CCHD Screening
Double outlet right ventricle
Effect of CCHD on O2 Saturations
Modified diagrams courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.
www.cdc.gov/ncbddd/heartdefects
Effect of CCHD on O2 Saturations
Modified diagrams courtesy of the Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities.
www.cdc.gov/ncbddd/heartdefects
5
Thangaratinam et al., 2012.
Reliability of Pulse Ox Screening for CCHD
Metaanalysis of 13 eligible studies with data for 229,421 newborn babies Overall sensitivity of pulse oximetry for detection of critical congenital heart defects was 76.5% (95% CI 67.7 – 83.5)
Specificity was 99.9% (95% CI 99.7 – 99.9) Falsepositive rate of 0.14% (95% CI 0.06 – 0.33) Low false positive rate when pulse ox done after 24 hours from birth compared to before 24 hours
New Jersey Critical Congenital Heart Defects Screening Program
CCHD Screening 2011
Indiana Law Enacted
1 st Newborn
Kemper Pediatrics Paper
New Jersey Law Enacted
AAP CCHD Screening Map: 2011 State Actions on CCHD
3 states enacted legislation
New Jersey Critical Congenital Heart Defects Screening Program
AAP CCHD Screening Map: States’ Actions During 2014 (As of January 29, 2015)
New Jersey Critical Congenital Heart Defects Screening Program
Performing the Screen
Best results when infant is at least 24 hours of age
Use proper sensor for the device
Conduct screening in quiet area and, if possible, with parent present to soothe and comfort the infant
Conduct screening while infant is awake and quiet
Avoid screening when infant is crying, cold or in a deep sleep
New Jersey Critical Congenital Heart Defects Screening Program
Reich et al., 2008.
Motion artifact
Ambient light
NJ Recommended Algorithm: Abridged Version
95%100% in both extremities AND a difference of 3% or less between the readings. 95%100% in both extremities AND a difference of 3% or less between the readings.PASS
90%94% in either extremity OR a difference of 4% or more between the readings. Rescreen the infant up to 2 times, for a total of 3 screens.
90%94% in either extremity OR a difference of 4% or more between the readings. Rescreen the infant up to 2 times, for a total of 3 screens.
RESCREEN IN 1 HOUR
90%94% in either extremity OR a difference of 4% or more between the readings after three screens.
90%94% in either extremity OR a difference of 4% or more between the readings after three screens.
FAIL
A reading of 89% or less in either extremity.A reading of 89% or less in either extremity. IMMEDIATE
FAIL
Evaluation for Failed Screen
Complete echocardiogram
New Jersey Critical Congenital Heart Defects Screening Program
Managing the Failed Screen
Unless a noncardiac cause can be identified for a failed screen, an infant who fails the screen should have a diagnostic echocardiogram done before being discharged
This could involve an echocardiogram within the hospital or birthing center, transport to another institution for the procedure, or the use of telemedicine for remote evaluation
New Jersey Critical Congenital Heart Defects Screening Program
Parent Education
Communication of Screening Results
Include screening results in discharge summary
Include in the handoff report to the receiving hospital if infant was transferred
Pediatrician
Early Intervention
Early Care and Education
First Office Visit
Pediatrician should have access to all screening results from hospital (Metabolic, CCHD, Hearing)
This should be a “clean handoff” between the hospital and pediatrician
If patient not appropriately screened at birth facility, develop strategies for screening and evaluation
New Jersey Critical Congenital Heart Defects Screening Program
Oster, 2013.
If Your Patient Failed CCHD Screening
Confirm that the infant had a diagnostic echocardiogram Make sure that the patient receives appropriate
followup, such as being seen by a cardiologist
Facilitate longterm followup for patients diagnosed with CCHDs
New Jersey Critical Congenital Heart Defects Screening Program
Signs & Symptoms of CHD
Lethargy
New Jersey Pulse Ox Screening Experience
8
New Jersey POxS Experience
Data Reporting Birthing facilities are required to report all failed POxS to the NJ Birth Defects Registry (BDR)
Health care professionals are required to report infants with CCHD (and other congenital defects) who are New Jersey residents to the BDR
Pulse ox core team and BDR staff investigate all POxS fails and registered cases of CCHD
New electronic birth record (Vital Information Platform) includes prenatal CCHD detection and POxS data
New Jersey Critical Congenital Heart Defects Screening Program
Results of NJ Screening
Number of livebirths: 313,005
Number of livebirths eligible to be screened: 304,211
Number of livebirths screened: 303,090
Proportion of eligible livebirths screened: 99.6 %
*Excludes expirations, <24 hours or not medically appropriate at end of period*Excludes expirations, <24 hours or not medically appropriate at end of period
New Jersey Critical Congenital Heart Defects Screening Program
Failed Screens Registered to NJBDR: August 31, 2011 September 30, 2014
One of 3 Criteria Prenatal diagnosis of
CCHD
Cardiac consult or echocardiogram planned prior to the screen
Total fails N= 208
N= 97
N= 111
New Jersey Critical Congenital Heart Defects Screening Program
Failed Screens Registered to NJBDR
Diagnostic evaluation attributable to POxS (n=97) 14 CCHD
12 CHD
27 PDA or PFO as only finding
35 – No documented reason for failed POxS 23 (66%) did not follow protocol
New Jersey Critical Congenital Heart Defects Screening Program
14 Infants with CCHD Were Detected
Coarctation of the aorta (5)
Ebstein anomaly (1)
Tricuspid atresia (1)
New Jersey Critical Congenital Heart Defects Screening Program
Characteristics of Failed Screens: CCHD CASE AGE AT
SCREEN PRE DUCTAL
FINAL DIAGNOSIS
1 2 DAYS 97 84 1 Y Y COARCTATION OF AORTA
2 3 DAYS 94 86 1 Y Y COARCTATION OF AORTA
3 2 DAYS 99 88 1 Y Y COARCTATION OF AORTA Out of state resident
4 2 DAYS 99 90 1 N Y COARCTATION OF AORTA
5 3 DAYS 92 97 3 Y Y COARCTATION OF AORTA* NICU
6 2 DAYS 43 39 1 Y Y DTGA
7 2 DAYS 85 85 1 Y Y DTGA
8 2 DAYS 87 84 1 Y Y TAPVR
9 2 DAYS 92 93 2 N Y TAPVR
10 2 DAYS 77 72 3 N Y TAPVR
11 3 DAYS 89 93 1 Y Y TAPVR
12 2 DAYS 85 88 1 Y Y TAPVR
13 2 DAYS 91 92 1 N Y TRICUSPID ATRESIA
14 3 DAYS 95 92 3 Y N EBSTEIN ANOMALY Passed national protocol
9
Other Conditions Detected
12 CHDs: Atrial septal aneurysm ASD VSD Pulmonary artery/pulmonary artery branch stenosis
9 Other significant nonCHD medical conditions: Sepsis Pneumonia Persistent pulmonary hypertension Pulmonary bulla
New Jersey Critical Congenital Heart Defects Screening Program
Screening Success
Since implementation, 99.6% of eligible infants were screened in New Jersey
POxS in NJ led to the detection of: 14 infants with critical congenital heart defects (CCHD)
12 infants with other congenital heart disease (CHD)
9 infants with serious noncardiac conditions
New Jersey Critical Congenital Heart Defects Screening Program
Acknowledgements
Lori Garg, MD, MPH
Mary Knapp, MSN, RN
Kim Van Naarden Braun, PhD
New Jersey Chapter, American Academy of Pediatrics
Regina Grazel, MSN, RN, BC, APNC
Fran Gallagher, MEd
Harriet Lazarus, MBA
Lindsay Caporrino, BS
Contact Information Regina Grazel, MSN, RN, BC, APNC
Project Coordinator, NJ DOH CCHD Screening Program [email protected]
[email protected]
American Academy of Pediatrics http://www.aap.org/enus/advocacyandpolicy/aaphealth initiatives/PEHDIC/Pages/NewbornScreeningforCCHD.aspx
New Jersey Chapter, American Academy of Pediatrics http://www.aapnj.org/showcontent.aspx?MenuID=1627